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Die ILAE-Klassifikation der Hippocampussklerose von 2013 im klinisch pathologischen Alltag

The 2013 ILEA classification of hippocampal sclerosis in the clinicopathological routine

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Zusammenfassung

Eine Hippocampussklerose (HS) ist durch subtotale Nervenzellverluste in verschiedenen anatomischen Sektoren des Hippocampus charakterisiert und praktisch immer mit einer medikamentös schwer behandelbaren Temporallappenepilepsie (TLE) vergesellschaftet. Die operative Entfernung des Hippocampus und angrenzender Strukturen des Schläfenlappens konnte in einer randomisierten klinischen Studie als erfolgreicher Therapieansatz bestätigt werden. Bei der histopathologischen Untersuchung finden sich jedoch unterschiedliche Verteilungsmuster hippokampaler Zellverluste. Wissenschaftliche Untersuchungen konnten diesen Mustern mit Einschränkung typische klinische Verläufe zuordnen. Die Internationale Liga gegen Epilepsie (ILAE) hat daher erstmalig 2013 eine internationale Konsensusklassifikation der HS vorgeschlagen. Wir erhoffen uns durch die Einteilung in 4 klinisch pathologische Subtypen eine bessere Aussagekraft zur Epilepsieentstehung und zu dem klinischen Verlauf. Es bleiben aber Fragen offen: 1. Warum führt ein Verlust erregender Nervenzellen im Hippocampus zu einer gesteigerten Erregbarkeit im Schläfenlappen? 2. Warum führen frühkindliche Schädigungen, wie beispielsweise Fieberkrämpfe, zur einseitigen Hippocampusschädigung? 3. Es erscheint paradox, dass die histopathologisch schwerste Form der HS mit subtotalen Nervenzelluntergängen in fast allen Hippocampusregionen den besten postoperativen Verlauf zeigt. 4. Es erscheint paradox, dass Patienten mit subtotalem Verlust von Pyramidenzellen in der CA1-Region und erhaltenem Körnerzellband präoperativ nur wenig messbare Gedächtnisstörungen aufweisen. Auf der Grundlage einer einheitlichen klinisch pathologischen Klassifikation soll uns die humane Hippocampusforschung zukünftig mehr Impulse zum Verständnis der Epilepsieentstehung und gleichsam Einblicke in Struktur und Biologie des Gedächtnisses liefern.

Abstract

Hippocampal sclerosis (HS) is characterized by segmental neuronal cell loss in different anatomical sectors oft he hippocampus and almost always associated with drug-resistant temporal lobe epilepsy. Surgical resection of the hippocampus and adjacent temporal lobe structures was proven to be the most successful treatment in a randomized clinical trial; however, histopathological examination of surgical specimens identified different patterns of hippocampal cell loss, which were associated with characteristic clinical presentations and postsurgical outcome. In 2013, the International League Against Epilepsy (ILAE) proposed an international consensus classification system of HS. The classification into four distinct clinicopathological subtypes may also help to promote further research efforts, as several intriguing questions are in need of clarification. 1. Why does loss of excitatory (pyramidal) neurons in the hippocampus lead to increased excitability of the temporal lobe? 2. Why do early childhood lesions, such as febrile seizures provoke unilateral damage of the hippocampus? 3. It seems also paradoxical that the histopathologically most severe form of HS (ILAE type 1) with neuronal cell loss in almost all hippocampal regions often has the best postsurgical outcome. 4. It seems paradoxical that patients with subtotal loss of pyramidal neurons in the CA1 region but an intact granular cell layer, preoperatively only have slight measurable memory impairment. Based on a uniform clinicopathological classification, it is hoped that research into the human hippocampus will provide more impulse in the undertstanding of the origins of epilepsy and simultaneously provide insights into the structure and biology of human memory.

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  1. Neuropathologisches Institut, Universitätskliniken Erlangen, Schwabachanlage 6, 91054, Erlangen, Deutschland

    Maria Wiegner &  Roland Coras

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  1. Maria Wiegner
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Correspondence to Roland Coras.

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M. Wiegner und R. Coras geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Wiegner, M., Coras, R. Die ILAE-Klassifikation der Hippocampussklerose von 2013 im klinisch pathologischen Alltag. Z. Epileptol. 30, 186–191 (2017). https://doi.org/10.1007/s10309-017-0117-2

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